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Naglazyme

galsulfase
Used for Inherited Metabolic Diseases, Lysosomal Storage Disease
Used for Inherited Metabolic Diseases, Lysosomal Storage Disease

Naglazyme (galsulfase) treats a rare condition called Mucopolysaccharidosis VI (or Maroteaux-Lamy syndrome). It's given as an infusion into the veins by a healthcare provider, so it's not a medication you can pick up at the pharmacy. This medication can cause reactions during or after the infusion including fever, chills, trouble breathing, and nausea.

Last reviewed on May 25, 2022
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What is Naglazyme (galsulfase)?

What is Naglazyme (galsulfase) used for?

  • Improve the ability of people with Mucopolysaccharidosis VI to walk and climb stairs

How Naglazyme (galsulfase) works

Naglazyme (galsulfase) is an enzyme replacement therapy (ERT), which means that it replaces an enzyme (protein) in your body. People with Mucopolysaccharidosis VI either don't have any or don't have enough of an enzyme that breaks down a certain sugar called glycosaminoglycan (GAG). GAG builds up in your body over time and can cause symptoms, such as bone changes, vision changes, and heart problems. Naglazyme (galsulfase) replaces the missing enzyme that breaks down the sugars, providing relief from some symptoms of Mucopolysaccharidosis VI.

Are you looking for information on Vimizim (elosulfase alfa) or Elaprase (idursulfase) instead?

Drug Facts

Common BrandsNaglazyme
Drug ClassEnzyme replacement therapy
Controlled Substance ClassificationNot a controlled medication
Generic StatusNo lower-cost generic available
AvailabilityPrescription only
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What are the side effects of Naglazyme (galsulfase)?

The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.

Common Side Effects

  • Infusion-related reactions (e.g., fever, chills, rash, trouble breathing, nausea; 56%)
  • Stomach pain (47%)
  • Joint pain (42%)
  • Ear pain (42%)
  • Pain (32%)
  • Rash (21%)
  • Pink eye (conjunctivitis) (21%)
  • Chills (21%)
  • Difficulty breathing (21%)

Other Side Effects

  • Headache
  • Nausea
  • Vomiting
  • Fever
  • Itchy skin
  • Chest pain
  • Sore throat
  • Stuffy nose
  • Hearing problems

Serious Side Effects

Contact your healthcare provider immediately if you experience any of the following.
  • Serious allergic reactions: swelling of the face, throat or tongue, wheezing, hives, rash, flushing, vomiting, trouble breathing

Source: DailyMed

The following side effects have also been reported

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor or nurse immediately if any of the following side effects occur:

Less common

Blurred or decreased vision

chest pain

difficult or labored breathing

dizziness

headache

hernia of the naval

nervousness

pounding in the ears

slow or fast heartbeat

swelling of the face

tightness in the chest

Incidence not known

Back pain

bluish lips or skin

confusion

cough

dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position

fever, chills, or sweating

hives or welts

joint pain

large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs

loss of bladder control

loss of bowel control

nausea or vomiting

paralysis of the limbs

stomach pain

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

Diarrhea

ear pain

loss of appetite

Less common

Body aches or pain

burning, dry, or itching eyes

congestion

dryness or soreness of the throat

excessive tearing

loss of or increase in reflexes

runny or stuffy nose

tender, swollen glands in the neck

trouble with swallowing

unusual tiredness or weakness

voice changes

Incidence not known

Difficulty with moving

loss of voice

muscle pain or stiffness

sneezing

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

pros-and-cons

Pros and cons of Naglazyme (galsulfase)

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Pros

The only medication FDA-approved for people with Mucopolysaccharidosis VI

Helps you walk farther and climb more stairs than before

Can possibly receive the infusion in your home if your provider thinks it's safe for you

Works well and is safe when used long-term

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Cons

Commonly causes infusion-related side effects (e.g., chills, itchy skin, and headache)

Infusion takes at least four hours

Only available as a brand medication so it can be expensive

Requires IV access, so it's not as convenient as taking a pill

pharmacist-tips

Pharmacist tips for Naglazyme (galsulfase)

pharmacist
  • Your infusion will take at least 4 hours to receive the full dose of Naglazyme (galsulfase). It can take longer depending on how well you tolerate the infusion. If you tolerate it well, you might be able to receive your dose at home. Talk to your provider to see if this is an option for you.

    • Stay on track with your infusions, even if you start to feel better. Naglazyme (galsulfase) will help make sure that glycosaminoglycan (GAG) doesn’t start to build up in your body again causing your symptoms to come back. If you miss a dose, be sure to reschedule your infusion right away.

      • If you have sleep apnea and use oxygen or a continuous positive airway pressure (CPAP) machine, bring it with you to your infusion. Your provider might also give you diphenhydramine (Benadryl) before your infusion that might make you sleepy. Bringing your oxygen or CPAP machine can be helpful if you have an infusion reaction or get too sleepy during your infusion.

        • Naglazyme (galsulfase) can cause infusion-related symptoms, such as feeling hot, itchy skin, headache, or nausea. Tell your provider if you're not feeling well during your infusion. They might give you medications like acetaminophen (Tylenol) or diphenhydramine (Benadryl) to help relieve these symptoms.

          • Serious allergic reactions (e.g., trouble breathing, hives, vomiting, or swelling of the face, throat, or tongue) might happen during or up to 24 hours after your Naglazyme (galsulfase) infusion. Let your provider know if you start to have these symptoms so that you can be treated immediately.

            faqs

            Frequently asked questions about Naglazyme (galsulfase)

            What is Mucopolysaccharidosis VI (or Maroteaux-Lamy syndrome)?
            Mucopolysaccharidosis VI is a rare, inherited condition where your body has lower levels of an enzyme, a specific protein, that breaks down a sugar called glycosaminoglycan (GAG). As a result, GAG builds up and causes cells in your body to get bigger. This then results in damage to many parts of your body, including the bones, lungs, and heart. Naglazyme (galsulfase) helps to replace this enzyme that you're missing so that your body can break down GAG and provide symptom relief.
            Do I pick up Naglazyme (galsulfase) at my regular pharmacy?
            No. Naglazyme (galsulfase) is considered a specialty medication, which means you will get it from a specialty pharmacy. Specialty pharmacies and infusion centers are used to dealing with medications like Naglazyme (galsulfase) that treat complicated or rare health conditions. The manufacturer has a savings program that might be able to offer resources to help you. Ask your healthcare provider or infusion center if you're interested in learning more.
            How long does a Naglazyme (galsulfase) infusion take?
            Naglazyme (galsulfase) infusions should take at least four hours, but it also depends on how fast Naglazyme (galsulfase) is infused into your veins. Your healthcare provider will decide the infusion rate based on your weight. How long each treatment takes also depends on how well you tolerate the infusion. If you're experiencing infusion-related reactions (e.g., chills, nausea, or trouble breathing), your provider might need to slow down or temporarily stop the infusion to manage your symptoms. This will make the infusion take longer to finish.
            How well does Naglazyme (galsulfase) work?
            Studies have shown that Naglazyme (galsulfase) works well to make it easier for people with Mucopolysaccharidosis VI to walk farther and climb more stairs. After just 6 months of using Naglazyme (galsulfase), people were able to walk 23% farther (in a 12-minute test) and were able to climb 38% more stairs (in a 3-minute test). Another study showed that Naglazyme (galsulfase) continued to improve endurance even after 10 years. If used at a young age, other possible benefits of using the medication include being able to breathe easier, growing taller, and slowing of bone- and heart-related problems.
            Is Naglazyme (galsulfase) safe for children?
            Yes. People begin to show signs and symptoms of Mucopolysaccharidosis VI in early childhood. Studies with Naglazyme (galsulfase) included people ages 5 to 29, with the majority being children. For younger children between ages 3 to 12 months, some studies have shown that it might be safe and well tolerated. Your child's provider will decide if Naglazyme (galsulfase) is right for them.
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            What are the risks and warnings for Naglazyme (galsulfase)?

            Naglazyme (galsulfase) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.

            risk-warning

            Serious allergic reactions

            Serious allergic reactions to Naglazyme (galsulfase) are possible. These reactions can include life-threatening problems like anaphylaxis (closing of the throat and difficulty breathing) and lower blood pressure. These reactions can happen during your infusion or up to 24 hours after your infusion has finished. Tell your provider right away if you begin to experience any of these symptoms during treatment. Your provider might stop the infusion and give you medications to help the symptoms go away. Your provider will decide if you should continue to receive Naglazyme (galsulfase).

            risk-warning

            Infusion-related reactions

            Your provider might give you medications before your Naglazyme (galsulfase) infusion to help avoid infusion-related reactions. Let your provider know if you start to feel unwell at any point during the infusion or after you receive treatment. In clinical studies, over half of people experienced infusion-related reactions, including fever, chills, rash, nausea, vomiting, and trouble breathing. And these reactions happened anytime from the first infusion up to almost 3 years later.

            If any of these reactions occur, your provider can adjust the infusion rate, temporarily stop the infusion, or give you medications to manage your symptoms. This might also mean you need to have medication before your next infusion to prevent or lessen the severity of these symptoms. If you've previously had an infusion-related reaction, your provider probably will want to observe you for a little while to see how you respond to the infusion in case they need to give you appropriate treatment.

            risk-warning

            Immune system reactions

            People can experience abnormal immune system reactions after enzyme replacement therapies like Naglazyme (galsulfase). These reactions cause parts of your body, like your kidneys, to become inflamed and damaged. This type of reaction, which typically happens after you have received at least one infusion, might not occur very quickly and can take several days to develop. Symptoms of these reactions can include fever, weight loss, or fatigue. If you have an immune system reaction, your provider will decide if you should continue receiving Naglazyme (galsulfase) infusions.

            risk-warning

            Heart or lung problems during infusion

            • Risk factors: Weighing 44 pounds or less | Heart or lung problems | Sleep apnea | Having a fever

              People who have certain heart or lung problems are at higher risk for experiencing serious breathing problems when receiving a Naglazyme (galsulfase) infusion. Your provider might want to monitor you for longer to make sure you tolerate the infusion. Also if you have sleep apnea, remember to bring your oxygen or CPAP machine. Tell your provider immediately if you experience any chest pain, have trouble breathing, or feel too sleepy during your infusion.

              risk-warning

              Spinal cord damage

              Spinal or cervical cord compression (SCC) can naturally occur in people with Mucopolysaccharidosis VI. But it's possible that Naglazyme (galsulfase) might cause SCC to happen quicker or become worse and require surgery. This is when your spinal or cervical cord is being compressed (or squeezed), which can lead to loss of feelings or nerve function in certain parts of your body. Let your provider know if you start having more back pain, loss of feeling in arms or legs, or you are not able to control when you need to go to the bathroom.

              dosage

              Naglazyme (galsulfase) dosage forms

              Typical dosing for Naglazyme (galsulfase)

              Naglazyme (galsulfase) is given as an infusion into your veins (intravenous or IV) by a healthcare provider. The dose is based on your body weight.
              The typical dose is 1 mg/kg once a week given IV.

              interactions

              Interactions between Naglazyme (galsulfase) and other drugs

              Naglazyme (galsulfase) may interact with certain medications or supplements. Always let your doctor and pharmacist know about any other medications or supplements (including prescribed and over-the-counter medications, vitamins, and dietary or herbal supplements) that you are currently taking. The list below does not include all possible drug interactions with Naglazyme (galsulfase). Please note that only the generic name of each medication is listed below.

              Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

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